Rare case of Rosai Dorfman disease involving paranasal sinuses in paediatric patient : a case report

Abstract EN

Rosai Dorfman disease (RDD) is a rare, benign disease of unknown aetiology.

It typically presents with massive, painless cervical lymphadenopathy but may have a varied presentation.

We report a paediatric case of RDD with initial isolated involvement of nasal cavity and PNS with subsequent involvement of cervical lymph nodes.

Endoscopic biopsy confirmed the pathological diagnosis and he was managed successfully with medical therapy.

At 20 month follow up there was no evidence of recurrence.

The ideal protocol for the treatment is still debated.

A long term follow-up is warranted to detect relapses.

This case report is illustrated aiming at developing insights into management and diagnosis of such rare clinical entity in a paediatric patient.